NGLY1 Specifically deglycosylates the denatured form of N-linked glycoproteins in the cytoplasm and assists their proteasome-mediated degradation. Cleaves the beta-aspartyl-glucosamine (GlcNAc) of the glycan and the amide side chain of Asn, converting Asn to Asp. Prefers proteins containing high-mannose over those bearing complex type oligosaccharides. Can recognize misfolded proteins in the endoplasmic reticulum that are exported to the cytosol to be destroyed and deglycosylate them, while it has no activity toward native proteins. Deglycosylation is a prerequisite for subsequent proteasome-mediated degradation of some, but not all, misfolded glycoproteins. Belongs to the transglutaminase-like superfamily. PNGase family. 5 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC 3.5.1.52; Hydrolase
Chromosomal Location of rat Ortholog: 15p16
Cellular Component:  cytoplasm; cytosol; nucleus
Molecular Function:  metal ion binding; peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase activity
Biological Process:  glycoprotein catabolic process; protein deglycosylation; protein quality control for misfolded or incompletely synthesized proteins
Reference #:  Q5XI55 (UniProtKB)
Alt. Names/Synonyms: N-glycanase 1; Ngly1; Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase; Peptide:N-glycanase; PNGase
Gene Symbols: Ngly1
Molecular weight: 74,677 Da
Basal Isoelectric point: 6.2  Predict pI for various phosphorylation states
Select Structure to View Below

NGLY1

Protein Structure Not Found.


Cross-references to other databases:  AlphaFold  |  STRING  |  BioGPS  |  Pfam  |  Phospho.ELM  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene